[Differential diagnosis of chylothorax in therapeutic practice].

The article is devoted to the differential diagnosis of chylous pleural effusion. The spectrum of traumatic and non-traumatic causes of chylothorax is discussed in detail. Examples of necessary diagnostic measures are given for the accumulation of milky fluid in the pleural cavity in order to verify chylothorax.

Functional insufficiency of the pancreas and the metabolic activity of the microbiota in cystic fibrosis adults patients.

AIM: In order to optimize the therapy, the functional state of the pancreas (P) and the peculiarities of metabolic activity of intestinal microbiota in adults with cystic fibrosis (CF) were assessed. MATERIALS AND METHODS: 14 CF patients (20-34 years, 7 men, 7 women) were enrolled. In 8 patients, the diagnosis was confirmed in the first year of life on the basis of clinical data, positive sweat test, 5 had genetic confirmation. In 4 patients, the diagnosis was confirmed at the age of 8-13 years and 2 patients aged 18, 27 years. In this group, genetic confirmation was in 4 subjects. In addition to general clinical studies, the level of C-peptide in blood, elastase and the concentration of short chain fatty acids in feces was determined. RESULTS: Of elastase feces in 9 patients was 5.5±4.7 icg/g, that is revealed severe exocrine insufficiency of the pancreas and in 5 patients the elastase level was normal and amounted to 402±124 icg/g. Deployed the clinical picture of diabetes mellitus was observed in 3 patients. Metabolic activity of the colon microflora as a whole was reduced, the sum of the concentration of short-chain fatty acids (ΣCn) was 6.03±4.11 mg/g at a rate of 10.61±5.11 (p<0.05). At the same time, in some patients (group 1, n=9), who were at the time of the study on antibiotic therapy, the value of ΣCn was 3.32±0.33 mg/g, and in patients receiving probiotic drugs (group 2, n=5), the activity of microflora did not differ from the norm ((ΣCn=11.03±2.01 mg/g). The correlation dependence of the ratio of the total iso-acids fraction in patients with MV to the normal values and the level of fecal elastase (r= -0.46, p=0.049) was revealed. CONCLUSION: Most patients with CF (64%) diagnosed with exocrine pancreatic insufficiency severe according elastase stool. The activity of faecal elastase correlated with parameters of microbiocenosis, which indicates the necessity of correction is not only functional insufficiency of the pancreas, but also the state of the microbiota.

[Enteral tube feeding in adult patients with cystic fibrosis and respiratory failure]. / Énteral'noe zondovoe pitanie u vzroslykh bol'nykh mukovistsidozom s dykhatel'noi nedostatochnost'iu.

AIM: To evaluate the efficiency of nocturnal hyperalimentation in adult patients with cystic fibrosis (CF) and respiratory failure. SUBJECTS AND METHODS: The investigation enrolled 17 patients older than 18 years (mean age, 25.6±4.2 years) diagnosed with very severe CF (forced expiratory volume in one second (FEV1), < 30%; body mass index (BMI), < 18.5 kg/m2); all the patients were on the waiting list for lung transplantation. Nutritional status and pulmonary function parameters, such as body weight, height, BMI, and FEV1, were measured at baseline, before and 6 and 9 months after tube feeding. RESULTS: The study group showed a considerable increase in body weight and BMI after 6 and 9 months. The change in lung function was statistically insignificant. Lung transplantation was successfully conducted in 5 patients; 4 died while on the waiting list; the cause of death was respiratory failure. CONCLUSION: Supplemental PEG tube feeding improves the nutritional status (BMI, body weight) of patients with very severe CF.

[Reliability of the Search for 19 Common Mutations in the CFTR Gene in Russian Cystic Fibrosis Patients and the Calculated Frequency of the Disease in Russian Federation].

A study of Russian cystic fibrosis (CF) patient DNA was conducted to assess the incidence frequency of 19 mutations, namely CFTRdele2,3(21kb), F508del, I507del, 1677delTA, 2143delT, 2184insA, 394delTT, 3821delT, L138ins, 604insA, 3944delGT, G542X, W128X, N1303K, R334W, and 3849+10kbC>T, S1196X, 621+1g>t, and E92K of the CFTR gene. We also sought to determine the estimated CF frequency in Russian Federation. In addition, we determined the total information content of the approach for 19 common mutations registration in the CFTR gene, 84.6%, and the allelic frequencies of the examined mutations: three mutations were observed with a frequency exceeding 5% (F508del, 53.98%, E92K, 6.47%, CFTRdele2,3(21kb), 5.35%); other mutations were observed with frequencies ranging from 0.13% to 3.0%. The CF population carrier frequency was 1 in 38 subjects, while the predicted CF frequency was 1 in 5776 newborns.

[Nutritional status in patients with cystic fibrosis: Possibilities of correction]. / Status pitaniia u bol'nykh mukovistsidozom: vozmozhnosti korrektsii.

The major risk factor for death in cystic fibrosis (CF) is a progressive lung injury; however, low nutritional status (NS) remains an important and underestimated problem in the management of these patients. The NS of a patient with CF is impaired by many factors, such as chronic malabsorption, pancreatic failure, chronic inflammation, and recurrent pulmonary infection - they all lead to higher energy demand in the presence of lower intake of nutrients. The NS of those with CF plays an important role in maintaining lung function. The patients with higher NS have longer life expectancies. According to the Russian National Registry, both pediatric and adult patients with CF have shorter life expectancies. The article discusses various nutritional support regimens and their impact on lung function parameters.

[ANTIBIOTICS SENSITIVITY OF STAPHYLOCOCCUS AUREUS, PSEUDOMONAS AERUGINOSA AND BACTERIA OF BURKHOLDERIA CEPACIA COMPLEX, PERSISTING IN LUNGS OF PATIENTS WITH MUCOVISCIDOSIS (MV)].

AIM: Study the spectrum of resistance to antibiotics and its variability of Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia complex (BCC), persisting in lungs of MV patients. MATERIALS AND METHODS: 312 strains of S. aureus, 213 strains of P. aeruginosa, 186 strains of BCC were studied. Monitoring of antibiotics sensitivity was carried out in strains, isolated from 30 patients with chronic S. aureus infection, from 22 patients with chronic BCC infection and from 21 patients with chronic pseudomonas infection. Interval of monitoring was from 14 days to 5 years 7 months. RESULTS: Study of S. aureus, P. aeruginosa and BCC strains has shown, that 35 and 33.3% of cases of staphylococcus infection, 37 and 46% of pseudomonas infection in children and adults, respectively, 100% of BCC infections were determined by multi-resistant clones. Study of genotypically identical strains, isolated from a single patient at different stages, has shown a change in antibiotics sensitivity as a result of persistence. CONCLUSION: Persisent infection of lungs in patients with MV is determined: by exchanging clones with varying antibiotics sensitivity or prolonged circulation of a single clone with a high degree of phenotypical and genotypical variability, that determine alteration of seeding of sensitive and resistant strains from the same patient during monitoring. This confirms the necessity of study of antibiotics sensitivity of strains for prescription of antibacterial therapy.